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Biopsy of the sural nerves distinguished two groups of patients with peroneal muscular atrophy (Charcot-Marie-Tooth): a hypertrophic type and a neuronal type. In patients with the hypertrophic type (10 nerves), 30-100 per cent of teased fibres of the sural nerve had demyelinated segments, numerous onion-bulb formations and often an increase in endoneurial space. Large and small fibres, with a diameter of more than 7 micron and less than 5 micron were diminished in number. Regeneration was scarce. There were more fibres with 60-120 myelin lamellae than in normal nerve, suggesting an atrophy of the axon. Biopsy of 19 sural nerves of patients with the neuronal type of PMA showed loss of large fibres (more than 7 micron in diameter). The number of small fibres was normal, presumably due to regeneration, since there were many "clusters" of small myelinated fibres. Fibres with demyelinated segments and onion-bulb formations were absent or rare and the endoneurial space was normal or slightly increased. Neither in the hypertrophic nor in the neuronal type did fibre loss occur selectively among the very largest fibres. Nine nerves from patients with hereditary spastic paraplegia and from a family with tremor and spasticity in addition to PMA showed changes similar in type but often milder in degree than nerves of the neuronal type of PMA. The number of unmyelinated fibres was normal in 12 of 21 nerves from patients with PMA; it was increased in 5 and diminished in 3 nerves.
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PMID:Peroneal muscular atrophy (PMA) and related disorders. II. Histological findings in sural nerves. 86 16

ALS is the most common of the various MNDs, which also include the clinical entities of PBP, PMA, and PLS. Mean age of onset of ALS is 57 years, and there is a sex predilection for men in a ratio of 1.5:1. Area of first symptom is in the lower extremity is 36 per cent of cases, in the upper extremity in 32 per cent, and 25 per cent of patients have a bulbar onset. Motor cranial nerves in the lower brain stem from cranial nerve nuclei or corticobulbar tract degeneration are affected. This results in symptoms of speech and swallowing difficulty and emotional lability in up to 60 per cent of cases. One hundred per cent of cases have motor weakness, over 90 per cent have muscle atrophy and fasciculations, and 47 per cent have spasticity. There seems to be a pattern of progression of ALS signs and symptoms based on area of onset with LLE involvement tending to follow RLE weakness, LUE weakness following RUE onset, and RUE involvement following next in patients whose onset is bulbar. Significant numbers of ALS patients had sparing of involvement of parts of the body for follow-up times approaching 3 years. Although the majority of patients experience a deterioration that is significantly linear, seven of 28 patients or 25 per cent achieved a plateau lasting a minimum of 9 months. Survival in our series was 4.08 years for all forms of MND.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Amyotrophic lateral sclerosis. Its natural history. 356 82